Journal of Rare Cardiovascular Diseases
ISSN: 2299-3711 (Print)
e-ISSN: 2300-5505 (Online)
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Unmasking Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA): A Rare Paediatric Cardiomyopathy Presentation and Its Diagnostic Challenges
Dr Ashok Rai
Dr Alok C Bhardwaj
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1
Heritage Institute of Medical Sciences, Varanasi
2
Popular Superspeciality Hospital, Varanasi.
Received: 2020-01-07
Revised: 2020-02-10
Accepted: 2020-02-25
Published: 2020-03-18
Full Text
Abstract
|Introduction: ALCAPA is a very rare congenital coronary anomaly, representing a potentially reversible cause of cardiomyopathy in the paediatric age group. Its nonspecific clinical presentation may be the reason why diagnosis is often delayed until advanced heart failure is manifested. Methods: A retrospective observational study was carried out over a 5-year period in a tertiary referral hospital. Paediatric patients with a confirmed diagnosis of ALCAPA were included. Data on clinical presentation, referral diagnoses, electrocardiographic and echocardiographic findings, surgical management, and follow-up outcomes were analysed. Heart failure severity was determined using the Ross classification, and left ventricular systolic function was assessed echocardiographically. Results: Twenty two children were included, with 73% presenting within the first year of life (median age 4 months). At presentation, 89% had overt heart failure, and referral diagnoses were frequently nonspecific. Transthoracic echocardiography established the diagnosis in 75.8% of cases, often relying on indirect markers. Severe left ventricular dysfunction and significant mitral regurgitation were common. Surgical correction was performed in 76.6% of patients, predominantly by direct reimplantation of the left coronary artery into the aorta. Significant improvement in left ventricular systolic function was observed during follow-up (p = 0.014). Overall mortality was 22%, largely among patients presenting with advanced disease or delayed intervention. Conclusion: ALCAPA continues to predominantly present in infancy with severe heart failure coupled with a diagnostic delay. Early diagnosis and timely surgical revascularization hold the key to improving outcomes in this rare but treatable pediatric cardiomyopathy.
Keywords
Anomalous left coronary artery from the pulmonary artery; Pediatric cardiomyopathy; Congenital coronary anomaly; Echocardiography; Surgical revascularization.
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Keywords
Classification of Rare Cardiovascular Diseases
anticoagulation
atrial fibrillation
atrial septal defect
cardiomyopathy
computed tomography
congenital heart disease
echocardiography
electrocardiogram
electrocardiography
heart failure
implantable cardioverter‑defibrillator
magnetic resonance imaging
pregnancy
pulmonary arterial hypertension
pulmonary hypertension
rare cardiovascular disease
rare disease
right heart catheterization
right ventricular failure
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